title: Liver transplantation in patients with cystic fibrosis: analysis of United Network for Organ Sharing data.
abstract: The improved life expectancy of patients with cystic fibrosis (CF) has led to a change in the impact of liver disease on the prognosis of this population. Liver transplantation has emerged as the procedure of choice for patients with CF and features of hepatic decompensation and for intractable variceal bleeding as a major manifestation. We retrospectively reviewed the United Network for Organ Sharing database to analyze the outcomes of 55 adults and 148 children with CF who underwent liver transplantation, and we compared them to patients who underwent transplantation for other etiologies. We additionally compared the benefits of liver transplantation among patients who underwent transplantation for cystic fibrosis-related liver disease (CFLD) and those who remained on the waiting list. The 5-year survival rates for children and adults undergoing liver transplantation were 85.8% and 72.7%, respectively (P = 0.016). A multivariate Cox regression analysis comparing pediatric and adult CF patients to patients who underwent transplantation for other etiologies noted lower 5-year survival rates (P < 0.0001). However, compared to those remaining on the waiting list, pediatric transplant recipients with CF (hazard ratio = 0.33, 95% confidence interval = 0.16-0.70, P = 0.004) and adult transplant recipients with CF (hazard ratio = 0.25, 95% confidence interval = 0.11-0.57, P = 0.001) gained a significant survival benefit. In conclusion, long-term outcomes in patients with CFLD are acceptable but are inferior in comparison with the outcomes of those undergoing transplantation for other etiologies. Despite such observations, a survival benefit was noted in transplant patients versus those who remained on the waiting list.
authors.affiliation: Unidad de Hígado y Trasplante Hepático, Hospital Universitario Austral, Pilar, Argentina. mmendiza@cas.austral.edu.ar
authors.forename: Manuel
authors.initials: M
authors.lastname: Mendizabal
authors.forename: K Rajender
authors.initials: KR
authors.lastname: Reddy
authors.forename: James
authors.initials: J
authors.lastname: Cassuto
authors.forename: Kim M
authors.initials: KM
authors.lastname: Olthoff
authors.forename: Thomas W
authors.initials: TW
authors.lastname: Faust
authors.forename: George A
authors.initials: GA
authors.lastname: Makar
authors.forename: Elizabeth B
authors.initials: EB
authors.lastname: Rand
authors.forename: Abraham
authors.initials: A
authors.lastname: Shaked
authors.forename: Peter L
authors.initials: PL
authors.lastname: Abt
mesh_terms: D000293:Adolescent; D000328:Adult; D016009:Chi-Square Distribution; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000401:mortality; D058625:End Stage Liver Disease / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors; D009927:Tissue and Organ Procurement; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D014850:Waiting Lists / Q000401:mortality; D055815:Young Adult